INTRODUCTION: Darier white disease is determined by autosomal gene
or it is determined by mutation of gene. (1) The follicular and
non-follicular hyperkeratotic papules with waxy scales are constant
features. However the disorder rarely presents along with a myriad of
unusual cutaneous lesions. Linear, unilateral, bullous cornifying or
solitary hypertrophic lesions are some such varieties. (2) We are
reporting a case of Darier's disease presented with condylomata
accuminata like growth over external genitalia for its rarity.

CASE REPORT: A 35 year old female born to non-consanguineous
marriage, presented with skin lesions of 15 years duration, consisting
of multiple brown black, warty, greasy, follicular and non-follicular
papules, some of which also showed keratin plugs, mainly over the
seborrhoeic areas, few papules were also present over the dorsa of
fingers, hands, feet and both shins. Red and white bands and V shaped
nicking at the free margins were noted on the finger nails.
Hyperkeratotic verrucous (Warty) growth present over the external
genitalia and preianal area, few areas showing erosions. [Fig. 1 &
Fig. 2]

Routine investigations were within normal limits. VDRL, HIV1 &
2 and HbsAg were negative. Punch biopsy from the lower back and excision
biopsy from external genitalia were showed hyperkeratosis, suprabasal
lacunae, corps-ronds and grains and hyperkeratotic invagination filled
with keratinous material and acantholytic cells suggestive of
Darier's disease. [Fig. 3]

DISCUSSION: The disease was first reported independently by Darier
and White in 1889. Darier's disease (DD) is a rare congenital
acantholytic disorder transmitted as an autosomal dominant trait. It may
also occur as mutation. Both sexes are affected with equal frequency. It
usually begins in childhood but can manifest at any time. (3)

Mutation in the gene ATP2A2 cause Darier's disease. ATP2A2
located on 12q23-24, encodes the endoplasmic reticulum Ca 2+ ATP, which
is a calcium pump. This pump maintains low cytoplasmic Ca2+ve level by
actively transporting Ca ions from the cytosol in to the lumen of the
endoplasmic reticulum. (4)

The lesion may first appear as skin colored or yellow brown papules
with a greasy, warty texture. The lesion are especially common in
seborrhoeic areas such as the forehead, scalp, margin of the scalp,
nasolabial folds, ears, chest and back. In less than 10% of patients,
flexural disease predominates, with large, warty, vegetative papules in
the axillae, groins or perineum. Frequently associated with pruritus.
Heat, sweat, humidity, sunlight, UVB exposure, lithium, oral
corticosteroids and mechanical trauma have been reported to exacerbate
this condition. (6)

Involvement of hands include punctate keratosis, palmar pits and
haemorrhagic macules, acrokeratosis verruciformis like lesions are
present in half the patients. Nail changes like white and red
longitudinal bands, longitudinal ridges, V shaped nick and subungual
hyperkeratosis are frequently found. (7) Mucosal lesions appear as white
papules with a central depression (cobblestone) lesions commonly seen in
the mouth, but can also occurs in genital mucosa. (8)

The histopathological finding, irrespective of clinical
presentation, characterized by focal changes of acantholysis and
presence of dyskeratotic cells resembling Corps ronds and grains
associated with suprabasal clefts around the preserved villi,
hyperkeratosis and parakeratosis. These findings are classical of
Darier's disease, but are also found in Hailey Hailey disease,
Grover's disease and warty dyskeratoma. (9)

In 1984, Chorzelski et al reported a 23-year old female with
multiple asymptomatic dome-shaped papules on the labia majora.
Clinically, this was different from Hailey-Hailey disease in that
papules were persistent for more than six years and there were no
vesicles, fissuring and erosions. Histology showed hyperkeratosis,
parakeratosis with some areas showing intraepidermal vesiculation and
acantholysis similar to Hailey-Hailey disease, while other areas were
more like Darier's disease showing dyskeratotic cells throughout
the epidermis, including Corps ronds and grains. Skin and serum
immunofluorescence were negative. The author regarded this as a new
entity and named the disease as Papular acantholytic dyskeratosis (PAD).

PAD is an uncommon disorder of the genitalia, usually asymptomatic
but sometimes may be pruritic. It is more commonly reported in females,
with lesions occurring on the vulva and perianal area, but there has
also been a report of a male patient with lesions over the penile shaft.
(10) Clinically, PAD must be differentiated from genital papules of
infectious origin, Hailey-Hailey disease, localized Darier's
disease of genital area and various pre-malignant and malignant
conditions. (9)

In our case female patient presented with condyloma accuminata like
growth over external genitalia and perianal area, which is rare clinical
morphology of Darier's disease and confirmed by histological
findings. It is clinically differentiated by PAD and histologically by
warty dyskeratoma. We are reporting this case for its morphological
rarity.